Anti-Filamin B/FLNB Antibody Picoband® (monoclonal, 11E2D2)

SKU: M02562-1

Size: 100 μg

Storage: At -20℃ for one year from date of receipt. After reconstitution, at 4℃ for one month. It can also be aliquotted and stored frozen at -20℃ for six months. Avoid repeated freezing and thawing.

Form: Lyophilized

Reactivity: Human

Applications: WB,IHC,ICC,IF

Application Details: Western blot, 0.25-0.5 μg/ml, Human
Immunohistochemistry(Paraffin-embedded Section), 2-5 μg/ml, Human
Immunocytochemistry/Immunofluorescence, 5 μg/ml, Human

Gene Name: FLNB

Specificity:

Background: Filamin B, beta (FLNB), also known as Filamin B, beta (truncated actin binding protein 278 homolog), is a cytoplasmic protein which in humans is encoded by the FLNB gene. This gene encodes a member of the filamin family. The encoded protein interacts with glycoprotein Ib alpha as part of the process to repair vascular injuries. The platelet glycoprotein Ib complex includes glycoprotein Ib alpha, and it binds the actin cytoskeleton. Mutations in this gene have been found in several conditions: atelosteogenesis type 1 and type 3; boomerang dysplasia; autosomal dominant Larsen syndrome; and spondylocarpotarsal synostosis syndrome. Multiple alternatively spliced transcript variants that encode different protein isoforms have been described for this gene.

Immunogen: E.coli-derived human Filamin B/FLNB recombinant protein (Position: Q397-D701).

Clonality: Monoclonal

Contents: Each vial contains 4 mg Trehalose, 0.9 mg NaCl and 0.2 mg Na2HPO4.

Purification: Immunogen affinity purified.

Reconstitution: Adding 0.2 ml of distilled water will yield a concentration of 500 μg/ml.

Reference: 1. Bicknell, L. S., Farrington-Rock, C., Shafeghati, Y., Rump, P., Alanay, Y., Alembik, Y., Al-Madani, N., Firth, H., Karimi-Nejad, M. H., Kim, C. A., Leask, K., Maisenbacher, M., and 14 others. A molecular and clinical study of Larsen syndrome caused by mutations in FLNB. J. Med. Genet. 44: 89-98, 2007. 2. Bicknell, L. S., Morgan, T., Bonafe, L., Wessels, M. W., Bialer, M. G., Willems, P. J., Cohn, D. H., Krakow, D., Robertson, S. P. Mutations in FLNB cause boomerang dysplasia. J. Med. Genet. 42: e43, 2005. Note: Electronic Article. 3. Biesecker, L. G. Phenotype matters. Nature Genet. 36: 323-324, 2004.

Uniprot ID: O75369

Host: Mouse

Concentration: Adding 0.2 ml of distilled water will yield a concentration of 500 μg/ml.

Conjugate:

Cross Reactivity: No cross-reactivity with other proteins.

Isotype: Mouse IgG1

Phospho_site:

Clone Number: Clone: 11E2D2

Observed Molecular Weight: 278 kDa

Calculated Molecular Weight:

Gene ID: 2317

Protein Name: Filamin-B

Gene Full Name: filamin B

Synonyms: ABP 278; ABP 280 homolog; Actin binding like protein; AOI; Beta filamin; FH1; Filamin 3; Filamin B; Filamin homolog 1; FLN B; FLN1L; FLN3; FLNB; LRS1; SCT; TABP; TAP; Thyroid autoantigen; Truncated ABP